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calciphylaxis; uremic calcific arteriopathy/arteriolopathy

Etiology: - hypercalcemia of any cause - most common in patients with a) end-stage renal disease & secondary hyperparathyroidism b) post-renal transplantation patients - thrombophilia/hypercoagulable state frequently involved [12] - lupus anticoagulant - antithrombin deficiency - protein C deficiency - protein S deficiency - warfarin may play role by reducing plasma protein C & plasma protein S Epidemiology: 1) rare 2) may occur more frequently in females Pathology: 1) systemic medial calcification of medium-size arteries 2) tissue calcification 3) hyperparathyroidism, vitamin D supplementation, hyperphosphatemia play a role 4) factors associated with uremia play a role may contribute 5) histopathology a) arterial occlusion & calcification b) absence of vasculitis c) calcifications in subcutaneous tissue within necrotic lipocytes & within walls of small blood vessels [7] d) confluent epidermal necrosis [7] * histopathology images [9] Clinical manifestations: 1) precipitation by a specific event, such as skin trauma or injection 2) painful subcutaneous nodules or plaques with red-brown discoloration (extremely painful) [7] 2) areas of ischemic necrosis develop in dermis, subcutaneous fat, & less often muscle 3) livedo reticularis develops 4) painful &/or purpuric subcutaneous nodules &/or plaques with red-brown discoloration develop on areas with most adipose tissue: buttocks, trunk, breast, proximal extremities - may form bullae 5) progression of nodules & plaques to ischemic/necrotic ulcers 6) black eschar formation in more advanced cases 7) infection & superinfection of ulcers 8) lesions may also appear on hands, fingers & lower extremities * images [9] Laboratory: 1) no diagnostic tests - elevated serum PTH, serum phosporous, serum Ca+2 may be observed, but these are not always present - elevated serum calcium x serum phosphate product 2) skin biopsy Radiology: - plain radiographs, computed tomography may be useful - bone scintigraphy may be useful * radiograph of hand [9] Complications: 1) high mortality (up to 58%) due to infection 2) 1 year survival is 37% [12] 2) ischemic myopathy is uncommon Differential diagnosis: 1) peripheral vascular disease 2) vasculitis 3) emboli 4) cryoglobulinemia 5) warfarin necrosis 6) cellulitis 7) nephrogenic systemic fibrosis Management: 1) aggressive wound care 2) pain management - hydromorphone is metabolized by livec, cleared by hemodialysis [7] 3) correction of Ca+2 & inorganic phosphate a) cessation of vitamin D supplementation b) treatment hyperphosphatemia with saline & sevelamer (RenaGel) rather than calcium acetate (PhosLo) 4) sodium thiosulfate may be beneficial in some patients [7,12] 5) cinacalcet has been successfully used [2] 6) avoidance of local tissue trauma - injections, blood transfusions 7) dialysis as needed 8) consider withdrawal of immunosuppression in renal transplant patients refractory to interventions 9) hyperbaric oxygen may be of some benefit [7,12] 10) tissue plasminogen activator [12] 11) parathyroidectomy [2,12] 12) surgical debridement [12] 13) prognosis is poor; response to therapy is uncertain

General

metastatic calcification sign/symptom

References

  1. UpToDate 11.2 2003 http://www.uptodate.com
  2. Essary LR and Wick MR Cutaneous calciphylaxis. An underrecognized clinicopathologic entity. Am J Clin Pathol 2000, 113:280 PMID: 10664631
  3. Arch-Ferrer et al, Therapy for calciphylaxis: an outcome analysis. Surgery 2003, 134:941 PMID: 14668726
  4. Janigan DT et al, Calcified subcutaneous arterioles with infarcts of the subcutis and skin ('calciphylaxic') in chronic renal failure. Am J Kidney Dis 200, 35:588 PMID: 10739777
  5. Velasco N et al, Successful treatment of calciphylaxis with cinacalcet - An alternative treatment to parathyroidectomy? Nephrol Dial Transplant 2006, 21:1999 PMID: 16603572
  6. Kale EK Metastatic calcification Dermatology Online Journal 9(4):2 , 2001 http://dermatology.cdlib.org/94/NYU/Nov2001/3.html
  7. Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2021.
  8. Weenig RH, Sewell LD, Davis MD, McCarthy JT, Pittelkow MR. Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol. 2007 Apr;56(4):569-79. Epub 2006 Dec 1. PMID: 17141359
  9. Nunley JR, Elston DM (images) Medscape: Calciphylaxis http://emedicine.medscape.com/article/1095481-overview
  10. DermNet NZ: Calciphylaxis (images) http://www.dermnetnz.org/systemic/calciphylaxis.html
  11. Ross EA Evolution of treatment strategies for calciphylaxis. Am J Nephrol. 2011;34(5):460-7 PMID: 21986387
  12. McCarthy JT, El-Azhary RA, Patzelt MT et al Survival, Risk Factors, and Effect of Treatment in 101 Patients With Calciphylaxis. Mayo Clin Proc. 2016 Oct;91(10):1384-1394. PMID: 27712637 - El-Azhary RA, Patzelt MT, McBane RD et al Calciphylaxis: A Disease of Pannicular Thrombosis. Mayo Clin Proc. 2016 Oct;91(10):1395-1402. PMID: 27712638
  13. Nigwekar SU, Thadhani R, Brandenburg VM. Calciphylaxis. N Engl J Med 2018; 378:1704-1714. May 3, 2018 PMID: 29719190 https://www.nejm.org/doi/full/10.1056/NEJMra1505292
  14. Nigwekar SU, Kroshinsky D, Nazarian RM et al Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis. 2015 Jul;66(1):133-46. Review. PMID: 25960299 Free PMC Article